POEMS syndrome: studies in a patient with an IgG-kappa M protein but no polyneuropathy.
A 48-year-old woman had a variation of the syndrome of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the so-called POEMS syndrome). The patient's neurological findings were entirely normal, but she had splenomegaly, hyperprolactinemia with galactorrhea and oligomenorrhea, a thyroid nodule with evidence of mild thyroiditis on aspiration biopsy specimen, and IgG-kappa monoclonal gammopathy, and hyperpigmentation and thickening of the skin. A short course of plasmapheresis (twelve 4-L exchanges in one month) did not alter any of the clinical abnormalities, but did result in a 70% decrease in the monoclonal IgG level (from 2.2 to 0.7 g/dl).
D001707:Biopsy, Needle; D001798:Blood Proteins / Q000032:analysis*; D004700:Endocrine System Diseases / Q000175:diagnosis; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D007074:Immunoglobulin G; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007136:Immunoglobulins; D008875:Middle Aged; D010859:Pigmentation Disorders / Q000175:diagnosis; D010956:Plasmapheresis; D011115:Polyneuropathies / Q000175:diagnosis; D012871:Skin Diseases / Q000175:diagnosis; D013163:Splenomegaly; D013577:Syndrome; D013959:Thyroid Diseases / Q000175:diagnosis
D001798:Blood Proteins; D007074:Immunoglobulin G; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007136:Immunoglobulins; C009823:M-proteins (Myeloma)